皮肤性肝卟啉症中的血红素生物合成：与酒精中毒和肝脏疾病的比较。

Haem biosynthesis in cutaneous hepatic porphyria: comparison with alcoholism and liver disease.

作者信息

Brodie M J, Thompson G G, Moore M R, McColl K E, Goldberg A, Hardie R A, Hunter J A

出版信息

Acta Hepatogastroenterol (Stuttg). 1979 Apr;26(2):122-8.

Abstract

The enzymes of haem biosynthesis have been measured in the peripheral blood of 13 patients with cutaneous hepatic porphyria. The activity of leucocyte delta-aminolaevulinic acid synthase was significantly elevated (p less than 0.001) as was that of erythrocyte porphobilinogen deaminase (p less than 0.05). Leucocyte ferrochelatase activity was depressed (p less than 0.001) and the activity of erythrocyte uroporphyrinogen decarboxylase did not significantly differ from control values. Similar enzyme activities were assayed in 12 chronic alcoholics and 8 patients with liver disease and the results differed markedly from those obtained from the porphyric patients. It is unlikely that the raised leucocyte delta-amino-laevulinic acid synthase activity can be attributed to alcohol ingestion or liver disease. A defect in the activity of uroporphyrinogen decarboxylase may exist in cutaneous hepatic porphyria but this could not be demonstrated in erythrocytes in this study.

摘要

对13例皮肤型肝性卟啉病患者的外周血进行了血红素生物合成酶的检测。白细胞δ-氨基乙酰丙酸合成酶的活性显著升高（p<0.001），红细胞胆色素原脱氨酶的活性也显著升高（p<0.05）。白细胞亚铁螯合酶活性降低（p<0.001），红细胞尿卟啉原脱羧酶的活性与对照值无显著差异。对12例慢性酒精中毒患者和8例肝病患者进行了类似的酶活性检测，结果与卟啉病患者明显不同。白细胞δ-氨基乙酰丙酸合成酶活性升高不太可能归因于饮酒或肝病。皮肤型肝性卟啉病可能存在尿卟啉原脱羧酶活性缺陷，但在本研究中红细胞中未得到证实。